An enormous shunt between the portal and hepatic veins associated with multiple coronary artery fistulas.
A 9-year-old boy with a rare combination of multiple coronary artery fistulas and congenital shunt between the portal and hepatic veins (portal-systemic shunt) is presented. The most likely pathogenesis for the portal-systemic shunt in this case was persistence of the ductus venosus as a bypass tract of the portal vein. This shunt is considered as one cause of cardiomegaly and dilatation of the hepatic vein in this case, and careful follow-up is mandatory because this shunt could induce portal-systemic encephalopathy. There are a few reports of an anomalous portal venous connection to the hepatic vein as a result of abnormal portal embryogenesis. We experienced a unique case of congenital portal-systemic shunt associated with coronary artery fistulas. The present paper reports angiographic findings in this case and the etiological importance of the congenital portal-systemic shunt.
Mori
K
K
Department of Pediatrics, School of Medicine, University of Okayama, Japan.
Dohi
T
T
Yamamoto
H
H
Kamada
M
M
D009202:Cardiomyopathies; D002648:Child; D003330:Coronary Vessel Anomalies; D005402:Fistula / Q000151:congenital*; D006503:Hepatic Veins / Q000002:abnormalities*; D006801:Humans; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*